DOCSLIB.ORG

GP GUIDE to Pots (Postural Tachycardia Syndrome)

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
GP GUIDE to Pots (Postural Tachycardia Syndrome) GP GUIDE TO PoTS (Postural Tachycardia Syndrome) 1 WHAT IS PoTS? PoTS was characterised in 1993, but previously existed under various other names including irritable heart, soldier’s heart and idiopathic orthostatic intolerance. It is a heterogeneous group of disorders sharing similar characteristics. On assuming upright posture, there is an excessive increase in heart rate associated with symptoms of orthostatic intolerance and sympathetic over-activity. There is brain hypoperfusion, usually in the absence of hypotension. When humans adopt upright posture, approximately 500ml of blood drops into the abdominal cavity and limbs. A normal autonomic nervous system responds with immediate peripheral vasoconstriction and an increase in heart rate of up to 20bpm. In POTS, it is considered that vasoconstriction is inadequate, resulting in pooling of blood, relative hypovolaemia and reduced venous return to the heart. Heart rate, inotropic status and, in some patients, catecholamine levels increase further to compensate. Dizziness and syncope can occur in the presence of normal BP; in fact some patients with PoTS have a hypertensive response to standing. HOW COMMON IS PoTS? The incidence in the UK is unknown. However, it is probably under-diagnosed due to lack of awareness and non-specific symptomatology. It is five times more common in women and tends to affect people age 15 to 50. 2 SYMPTOMS OF PoTS Dizziness GI upset Syncope / pre-syncope Sweating Orthostatic headache Nausea Fatigue Insomnia Poor memory Weakness Poor concentration Visual greying or blurring Sense of anxiety Acrocyanosis (purplish hands/feet) Exercise intolerance Palpitations (tachycardia / ectopics) Tremulousness Neck/shoulder pain (muscle ischaemia) Patients may have some or all of the above symptoms. Most patients experience light-headedness, but 30% have blackouts. Symptoms tend to be worse on standing or prolonged sitting and exacerbated by heat, food and alcohol. Life expectancy is thought to be unaffected, but levels of disability can be equivalent to that found in congestive heart failure and COPD. WHAT CAUSES PoTS? PRIMARY PoTS Abrupt onset may follow infection, pregnancy, surgery, immunisation or trauma. There is some evidence of auto-immune aetiology. ‘Developmental’ PoTS affects teenagers (gradual onset around age 14 with symptoms peaking age 16) and 80% resolve within a few years. Hyperadrenergic PoTS – in some, a genetic defect has been identified SECONDARY PoTS Deconditioning (e.g. after prolonged bed rest following illness) Joint Hypermobility Syndrome (now considered by most authorities as indistinguishable from, if not identical to, the Ehlers-Danlos-hypermobility type, formerly known as EDS type III); an inherited multi-system connective tissue disorder. See www.hypermobility.org Other medical conditions – diabetes, amyloidosis, sarcoidosis, SLE * Poisons – alcohol, heavy metals * Cancer or chemotherapy 3 HOW IS IT DIAGNOSED? Diagnosis is usually made following a tilt table test. After resting, the patient is tilted head up by around 60 degrees for up to 45 minutes. In PoTS, heart rate increases by at least 30 bpm (40bpm in teenagers) or to >120 bpm. Symptoms are similar to those of hypotension and there is reduced cerebral perfusion, but by definition, blood pressure does not necessarily drop. However, there is an overlap with neurally mediated hypotension and 40% also have a drop in blood pressure. A stand test can also be used to diagnose PoTS. Pulse rate / BP can be measured supine then after 2, 5 and10 minutes of standing still. Look for acrocyanosis (puffiness and purplish discolouration of feet/hands) A sustained increase in heart rate of >30 bpm suggests PoTS. Stop if patient becomes very symptomatic. Some patients may faint during this test. Plasma noradrenaline levels are often elevated in the upright position in PoTS. Other pathology must be excluded and DIAGNOSTIC CRITERIA FOR underlying causes of PoTS considered. PoTS Depending on history and symptoms, tests 1. Sustained heart rate increase of 30 beats per minute or more within 10 may include FBC, biochemical profile, minutes of standing or head up tilt calcium, thyroid function, serum cortisol, (40 bpm age 12-19) in the absence of orthostatic hypotension catecholamine levels, urine HIAAs, ECG, 24 hour BP, ECG and EEG. 2. Standing heart rate is often 120 beats per minute or more within 10 minutes of standing or head up tilt 3. Orthostatic tachycardia may be accompanied by symptoms of cerebral hypo-perfusion and autonomic over-activity that are relieved by recumbence. 4. Criteria not applicable for low resting heart rate. 4 MANAGEMENT PHYSIOLOGICAL Increased fluid (2-2.5 litres/day) and salt intake (2 to 4g ie up to 2/3 level teaspoon, Not in hyperadrenergic PoTS) * - increases blood volume Graduated exercise programme – swimming is ideal - improves calf muscle pump Small frequent meals low in refined carbohydrates -reduces diversion of blood to gut. Elevate head of bed * - increases morning blood volume Avoid prolonged standing and sitting * – reduces venous pooling. Avoid heat and alcohol * – reduce vasodilatation. Support tights – class 3, waist high * - reduce venous pooling. Postural manoeuvres to avoid syncope eg elevate legs, cross legs and clench thighs and buttocks, clench a fist, rock up and down on tiptoes *- improves venous return. DRUG THERAPY Stop drugs that may cause orthostatic intolerance Considered drug therapy if 3 – 6 months of physiological measures are ineffective. Drugs used in PoTS are all unlicensed for this indication. Some hospitals offer a shared care agreement. Fludrocortisone Increases blood volume Midodrine Alpha -1 agonist / vasoconstrictor Beta blocker Reduce heart rate and improves diastolic filling Ivabradine Reduce heart rate and improves diastolic filling SSRIs / SNRIs Affects central control of HR and BP Clonidine Alpha-2 agonist / centrally sympatholytic Desmopressin Increases blood volume Methylphenidate Vasoconstricts Erythropoeitin Increase red cell mass/vasoconstricts Octreotide Splanchnic vasoconstrictor Pyridostigmine Facilitates ganglionic neural transmission CBT To help patients adjust to long-term illness and symptoms 5 WHY IS PoTS UNDERDIAGNOSED? Patients can repeatedly present with a multitude of symptoms including anxiety, often without clinical findings - typical ‘heart-sink’ patients. Patients may not recognise the significance of symptoms or be reluctant to divulge them for fear of the clinician’s response. Blood pressure and pulse recordings are usually taken with patients seated when recordings may be normal. There is little knowledge of PoTS within the medical community It is often misdiagnosed as anxiety, panic attacks, vasovagal syncope, chronic fatigue syndrome or inappropriate sinus tachycardia. WHY IS THIS IMPORTANT? Diagnosis of PoTS is commonly delayed by several years. PoTS causes considerable disability - patients can become wheelchair or bed bound. They are often unable to continue education or employment (25%) Treatments are available and 90% of patients will respond. Patients frequently receive psychiatric labels prior to correct diagnosis. Tools used to assess anxiety commonly rely on somatic anxiety symptoms (which may reflect high upright catecholamine levels). Using the Anxiety Sensitive Index (a cognitive-based measure of anxiety), PoTS patients tend to have lower anxiety levels than the general population. 6 WHAT SHOULD I DO IF I SUSPECT PoTS? 1. PoTS should be considered in patients (especially young women) with a combination of unexplained symptoms eg dizziness, syncope, fatigue, palpitations/tachycardia, headaches, exercise intolerance, anxiety. Have a high index of suspicion in diabetes, chronic fatigue syndrome and joint hypermobility syndrome. 2. Stand test - this can be undertaken in a GP consulting room. 3. Exclude other pathology with biochemistry tests (see above) and ECG (in syncope or family history of sudden death, exclude prolonged QT interval). 4. Consider 24 hour BP/ heart rate monitoring – normal recording does not exclude PoTS. (If equipment cannot be triggered by patient to record when symptomatic, PoTS, vasovagal syncope and orthostatic hypotension can be missed) 5. If PoTS is suspected, referral should be made to a physician with an interest in PoTS as diagnosis and management can be challenging. These consultants are often cardiologists with an interest in arrhythmias (i.e. cardiac electrophysiologists who may work in a syncope or blackout clinic). There are also some neurologists, medicine for elderly and neurovascular (autonomic) consultants with an interest in PoTS. There is a list of doctors with an interest in PoTS and syncope on the PoTS UK and STARS websites. (see below) Further information for clinicians, patients and carers is available from: www.potsuk.org www.stars.org.uk www.gpnotebook.co.uk http://www.patient.co.uk/health/postural-orthostatic-tachycardia-syndrome *currently no available medical evidence but generally accepted by experts. 7 Glossary for patients arrhythmia - abnormal heart rhythm auto-immune - when the immune system produces antibodies against the body’s own tissues autonomic - belonging to the nervous system that controls bodily functions that we do not have to think about catecholamine - a chemical produced by the adrenal gland CBT -cognitive behavioural therapy, a talking therapy cerebral perfusion - blood supply within the brain deconditioning - lack of physical fitness heterogeneous - varied or diverse hypoperfusion -reduced
Load more

Recommended publications
  • Orthostatic Intolerance V1 4.2.11, F10 Overview V2 6036-7,7038,7040,7042 Orthostatic Intolerance
    NASA-STD-3001 Technical Brief Orthostatic Intolerance V1 4.2.11, F10 Overview V2 6036-7,7038,7040,7042 Orthostatic Intolerance Executive Summary Orthostatic intolerance (OI) is an abnormal response to standing upright caused by an inability to maintain arterial blood pressure and perfuse cerebral tissue. It can result in presyncope and, ultimately, syncope (i.e. loss of consciousness). Specifically in the spaceflight community, OI is a major concern when crewmembers are re-introduced to gravity after landing due to decreased plasma volume and sympathetic nervous system dysfunction. OI must also be considered for standing crew experiencing acceleration loads. A variety of countermeasures can be implemented to reduce the symptoms of and/or completely mitigate OI. Standards Overview NASA-STD-3001 Vol. 1 4.2.11 Fitness-for-Duty Orthostatic Hypotension Standard (with Appendix F.10) All approved countermeasures (fluid loading and compression garments) shall be utilized to mitigate symptoms of orthostatic hypotension (presyncopal/syncopal symptoms) to prevent impacts to performance of critical mission tasks. NASA-STD-3001 Vol. 2 6.3.2.8 Fluid Loading Water Quantity for Earth Entry [V2 6036] 6.3.2.9 Crew recovery Water Quantity [V2 6037] 7.4.1 Physiological Countermeasures Capability [V2 7038] The system shall provide countermeasures to meet crew bone, muscle, sensory-motor, and cardiovascular requirements defined in NASA-STD-3001, Vol. 1. 7.4.3 Physiological Countermeasure Operations [V2 7040] The physiological countermeasure system design shall allow the crew to unstow supplies, perform operations, and stow items within the allotted countermeasure schedule. 7.4.5 Orthostatic Intolerance Countermeasures [V2 7042] The system shall provide countermeasures to mitigate the effects of orthostatic intolerance when transitioning from microgravity to gravity environments.
    [Show full text]
  • Neurocardiogenic Syncope and Associated Conditions: Insight Into Autonomic Nervous System Dysfunction
    Türk Kardiyol Dern Arş - Arch Turk Soc Cardiol 2013;41(1):75-83 doi: 10.5543/tkda.2013.44420 75 Neurocardiogenic syncope and associated conditions: insight into autonomic nervous system dysfunction Nörokardiyojenik senkop ve ilişkili durumlar: Otonomik sinir sistemi işlev bozukluğuna bir bakış Antoine Kossaify, M.D., Kamal Kallab, M.D. Department of Syncope and Electrophysiology, ND Secours/USEK University Hospital, Byblos, Lebanon Summary– Neurocardiogenic syncope is known to be asso- Özet– Nörokardiyojenik senkopun mekanizması hala tam ola- ciated with autonomic nervous system dysfunction, although rak aydınlatılamamasına ragmen otonom sinir sistemi işlev the mechanism has not been entirely elucidated. In this study, bozukluğu ile ilişkili olduğu bilinmektedir. Bu yazıda nörokardi- we sought to highlight the pathogenic role of the autonomic yojenik senkop patogenezinde otonom sinir sisteminin rolünü nervous system in neurocardiogenic syncope and to review destekleyen en göze çarpıcı konuları araştırırken, temelinde the associated co-morbidities known to have a dysautonomic otonom sinir sistemi bozukluğunun olduğu bilinen komorbid basis. Herein we discuss migraine, orthostatic hypotension, durumları da gözden geçidik. Bu amaçla otonom sinir siste- postural orthostatic tachycardia syndrome, endothelial dys- minin patogenezdeki rolü ve klinik çıkarımları üzerine odak- function, chronic fatigue syndrome, and carotid sinus hyper- lanarak migren, ortostatik hipotansiyon, postüral ortostatik sensitivity with a focus on the pathogenic role of the autonom- taşikardi sendromu, endotel işlev bozukluğu, kronik yorgunluk ic nervous system and any consecutive clinical implications. sendromu ve karotis sinüs hipersensitivitesi tartışıldı. Tilt testi Other conditions, such as pre-syncopal heart rate acceleration sırasında ortaya çıkabilen senkop öncesi kalp atımlarında hız- and/or instability and pre-syncopal breathing instability, which lanma ve/veya instabilite ve senkop öncesi instabil solunum occur during a tilt test, are discussed in the same perspective.
    [Show full text]
  • Ehlers-Danlos Syndrome and the Overlap with Orthostatic Intolerance
    10/9/2014 Ehlers‐Danlos Syndrome Ehlers‐Danlos Syndrome and the Overlap with Orthostatic Intolerance • Heterogeneous disorder of connective tissue • Characterized by varying degrees of: October 9, 2014 Skin hyperextensibility Joint hypermobility Cutaneous fragility Peter C. Rowe, MD •Most forms of EDS result from mutations in genes Sunshine Natural Wellbeing Foundation Professor of encoding fibrillar collagens or the collagen‐ Chronic Fatigue and Related Disorders modifying enzymes Johns Hopkins University School of Medicine 1. Royce PM, Steinmann B, Superti‐Furga A. The Ehlers‐Danlos syndrome. In: Connective Tissue and its Heritable Baltimore, USA Disorders. New York: Wiley‐Liss, 1993: 351‐407. 2. de Paepe A, Malfait F. The Ehlers‐Danlos syndrome, a disorder with many faces. Clin Genetics 2012;82:1‐11. Presenter Disclosure Information Ehlers‐Danlos Syndrome Peter C. Rowe, MD • Prevalence unknown, estimated at 1:5000 •Because fibrillar collagen provides strength and structure to essentially all tissues and organs, EDS •No relationships to disclose has widespread clinical manifestations •Early varicose veins, easy bruising •Easy fatigability and widespread pain common Royce PM, Steinmann B, Superti‐Furga A. The Ehlers‐Danlos syndrome. In: Connective Tissue and its Heritable Disorders. New York: Wiley‐Liss, 1993: 351‐407. EDS, JH, and Orthostatic Intolerance Classification of EDS Beighton P, et al. Am J Med Genetics 1998;77:31‐7. Overview of Ehlers‐Danlos Syndrome Classical Illustrative case (formerly EDS I and II) Orthostatic intolerance in EDS and JH Hypermobility (formerly EDS III) Challenges Vascular (formerly EDS IV) Kyphoscoliosis Arthrochalasia 3 generations with Classical EDS. Note hemosiderin deposition in knees and Dermatosparaxis shins, varicose vein stripping on R 1 10/9/2014 de Paepe A, Malfait F.
    [Show full text]
  • What Is the Autonomic Nervous System?
    J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.74.suppl_3.iii31 on 21 August 2003. Downloaded from AUTONOMIC DISEASES: CLINICAL FEATURES AND LABORATORY EVALUATION *iii31 Christopher J Mathias J Neurol Neurosurg Psychiatry 2003;74(Suppl III):iii31–iii41 he autonomic nervous system has a craniosacral parasympathetic and a thoracolumbar sym- pathetic pathway (fig 1) and supplies every organ in the body. It influences localised organ Tfunction and also integrated processes that control vital functions such as arterial blood pres- sure and body temperature. There are specific neurotransmitters in each system that influence ganglionic and post-ganglionic function (fig 2). The symptoms and signs of autonomic disease cover a wide spectrum (table 1) that vary depending upon the aetiology (tables 2 and 3). In some they are localised (table 4). Autonomic dis- ease can result in underactivity or overactivity. Sympathetic adrenergic failure causes orthostatic (postural) hypotension and in the male ejaculatory failure, while sympathetic cholinergic failure results in anhidrosis; parasympathetic failure causes dilated pupils, a fixed heart rate, a sluggish urinary bladder, an atonic large bowel and, in the male, erectile failure. With autonomic hyperac- tivity, the reverse occurs. In some disorders, particularly in neurally mediated syncope, there may be a combination of effects, with bradycardia caused by parasympathetic activity and hypotension resulting from withdrawal of sympathetic activity. The history is of particular importance in the consideration and recognition of autonomic disease, and in separating dysfunction that may result from non-autonomic disorders. CLINICAL FEATURES c copyright. General aspects Autonomic disease may present at any age group; at birth in familial dysautonomia (Riley-Day syndrome), in teenage years in vasovagal syncope, and between the ages of 30–50 years in familial amyloid polyneuropathy (FAP).
    [Show full text]
  • Orthostatic Intolerance and Syncope Associated with Chiari Type I Malformation O Prilipko, a R Dehdashti, S Zaim, M Seeck
    1034 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.2004.044636 on 16 June 2005. Downloaded from SHORT REPORT Orthostatic intolerance and syncope associated with Chiari type I malformation O Prilipko, A R Dehdashti, S Zaim, M Seeck ............................................................................................................................... J Neurol Neurosurg Psychiatry 2005;76:1034–1036. doi: 10.1136/jnnp.2004.048330 10 hours during which she presented the aforementioned The Chiari type I malformation (CM1) is characterized by presyncopal symptoms was unrevealing. The cardiac evalua- herniation of cerebellar tonsils to at least 3–5 mm below the tion included echocardiography and a 24 hour Holter plane of foramen magnum and can present with a wide recording and were both normal. A Doppler examination of variety of clinical symptoms, frequently including occipital exocranial and endocranial arteries was also normal. A headaches, secondary to bulbar and/or medullary distress. Schellong test (blood pressure (BP) and pulse monitoring Rarely, syncopal episodes have also been described and every minute for 3 minutes in the resting supine position, attributed to either compression of the midbrain ascending followed by standing up immobile and unsupported for up to reticular system, or vascular compromise (vertebrobasilar 20 minutes with BP and pulse monitoring every minute) artery compression, hypotension). We report the first case of demonstrated signs of orthostatic intolerance. BP increased a CM1 patient with frequently recurring syncope due to from 103/68 mmHg to 154/77 mmHg after movement from postural orthostatic tachycardia syndrome (POTS), a form of the horizontal to vertical position, associated with an increase orthostatic intolerance, whose symptoms resolved completely in heart rate (HR) from 73 to 111 beats/min (38 beats/min after surgical intervention.
    [Show full text]
  • Multiple System Atrophy – Cerebellar Type: Clinical Picture and Treatment of an Often- Overlooked Disorder
    Open Access Review Article DOI: 10.7759/cureus.10741 Multiple System Atrophy – Cerebellar Type: Clinical Picture and Treatment of an Often- Overlooked Disorder Juan Fernando Ortiz 1 , Sagari Betté 2 , Willians Tambo 3 , Feiyang Tao 4 , Jazmin Carolina Cozar 5, 6 , Stuart Isaacson 7 1. Neurology, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA 2. Neurology, Parkinson’s Disease and Movement Disorder Center of Boca Raton, Boca Raton, USA 3. Neurology, Universidad San Francisco de Quito, Quito, ECU 4. Neurology, School of Medicine, University of California, Irvine, Irvine, USA 5. Medicine, Universidad de las Américas, Quito, ECU 6. Family Medicine, Open Door Family Medical Center, Portchester, USA 7. Neurology, Parkinson's Disease and Movement Disorder Center of Boca Raton, Boca Raton, USA Corresponding author: Juan Fernando Ortiz, [email protected] Abstract Multiple system atrophy (MSA) is a rare, progressive, fatal, neurodegenerative disorder. There are two main types: the parkinsonian type (MSA-P) and cerebellar type (MSA-C). The disease usually presents with genitourinary dysfunction, orthostatic hypotension, and rapid eye movement (REM) sleep behavior disorder. Patients rapidly develop balance, speech, and coordination abnormalities. We present a review of the clinical picture and the actualized treatment modalities of the MSA cerebellar type. For the study methods, a PubMed search was done using the following medical subject headings (MeSH) terms: “multiple system atrophy/therapy". Inclusion criteria included studies in English, full papers, human studies, and publications in the last 30 years. Case reports and series were excluded. A total of 157 papers were extracted after applying the inclusion and exclusion criteria, and 41 papers were included for the discussion of this review.
    [Show full text]
  • Diagnostic Approach of Orthostatic Dizziness/Vertigo
    REVIEW ARTICLE Ann Clin Neurophysiol 2020;22(2):75-81 https://doi.org/10.14253/acn.2020.22.2.75 ANNALS OF CLINICAL NEUROPHYSIOLOGY Diagnostic approach of orthostatic dizziness/vertigo Hyung Lee, Hyun Ah Kim Department of Neurology, Keimyung University School of Medicine, Daegu, Korea This paper introduces new diagnostic criteria and differential diagnosis of orthostatic dizziness to help clinicians to diagnose hemodynamic orthostatic dizziness. Clinicians need to be able to discriminate hemodynamic orthostatic dizziness from other types of dizziness that are Received: August 14, 2020 induced or aggravated when standing or walking. Measurements of the orthostatic blood Revised: September 9, 2020 pressure and heart rate are important when screening hemodynamic orthostatic dizziness. Accepted: September 9, 2020 Detailed history-taking, a physical examination, and laboratory tests are essential for finding the cause of hemodynamic orthostatic dizziness. The differential diagnosis of hemodynamic orthostatic dizziness is crucial because it can be caused by various autonomic neuropathies. Key words: Orthostatic intolerance, Dizziness; Hemodynamic; Diagnosis; Orthostatic hypo- Correspondence to tension; Postural orthostatic tachycardia syndrome Hyun Ah Kim Department of Neurology, Keimyung University School of Medicine, 1035 Dalgu- beol-daero, Dalseo-gu, Daegu 42601, Korea Tel: +82-53-250-7475 Fax: +82-53-250-7840 INTRODUCTION E-mail: [email protected] Orthostatic dizziness is a common type of dizziness. The prevalence of orthostatic diz- ziness based on patients’ history varied from 2% to 57%.1-9 A population-based study9 ORCID found that the 1-year and lifetime prevalence rates of orthostatic dizziness were 10.9% Hyung Lee and 12.5%, respectively. Orthostatic dizziness commonly occurs in patients with orthostat- https://orcid.org/0000-0003-0568-6104 ic hypotension (OH) or postural tachycardia syndrome (POTS).10 Orthostatic dizziness oc- Hyun Ah Kim curs when the cerebral perfusion is critically reduced in patients with OH.
    [Show full text]
  • Managing Orthostatic Intolerance Peter Rowe, MD Johns Hopkins University School of Medicine
    Managing Orthostatic Intolerance Peter Rowe, MD Johns Hopkins University School of Medicine Moderated by Kim McCleary September 1, 2010 Hosted by the CFIDS Association of America Thank you for joining us! • 13th CFIDS Association webinar of 2010 series • Dr. Rowe and Kim are in different locations • 270 people preregistered to participate • Questions submitted with registration helped shape discussion topics • Time for Q&A after presentation – type them as you think of them them • Recording will be posted online within a couple of days • Dr. Rowe is not able to address individuals’ questions about their symptoms, test results or therapy What we learned from registrants: • 50% have been diagnosed with orthostatic intolerance (OI) • 30% have symptoms of OI but have not had testing • 60% indicate that OI has a significant impact on daily life • Only 5% indicate that it has little or no effect • 60% have participated in another Association webinar • Lots of questions submitted in advance! Peter M. Rowe, MD Johns Hopkins University School of Medicine Baltimore, Maryland Sunshine Natural Wellbeing Foundation Professor of Chronic Fatigue and Related Disorders Specializes in pediatric and adolescent medicine With colleague Hugh Calkins, credited with identifying link between CFS and OI Managing Orthostatic Intolerance CFIDS Association Webinar 1 September 2010 Peter C. Rowe, MD Sunshine Natural Wellbeing Foundation Professor of Chronic Fatigue and Related Disorders Johns Hopkins University School of Medicine Baltimore, USA Managing Orthostatic Intolerance • Introduction to the problem • Definition and overview of the physiology • Common forms of OI in CFS • Treatment of OI – Non-pharmacologic measures – Treating contributory conditions – Medications 15 year old with fatigue Insidious onset of fatigue and lightheadedness at age 13 Awakens unrefreshed after 10 hours of sleep.
    [Show full text]
  • Managing Orthostatic Intolerance in Adolescents
    Managing Orthostatic Intolerance in Adolescents Peter C. Rowe, M.D. Professor of Pediatrics Sunshine Natural Wellbeing Foundation Professor of Chronic Fatigue and Related Disorders Division of General Pediatrics and Adolescent Medicine Johns Hopkins University School of Medicine Abnormalities in the response to upright posture in those with orthostatic intolerance ↑ pooling, ↓ intra-vascular volume ↓ vasoconstriction Standing/ ↑ sympatho-adrenal Tilt test response ↓ NE/Epi ↑ NE/Epi NMH POTS Common Forms of OI • Initial Orthostatic Hypotension (IOH): – transient ↓ of 40 mm Hg in SBP or 20 mm Hg DBP within 15 sec of standing (more common in adolescents) • Orthostatic Hypotension (OH): – sustained ↓ of 20 mm Hg in SBP or 10 mm Hg in DBP within 3 min of standing or HUT (more common in older adults) • Delayed OH – OH occurring after 3 minutes upright Freeman R, et al. Consensus statement on the definition of orthostatic hypotension, neurally mediated syncope and the postural tachycardia syndrome. Clin Auton Res 2011;21:69-72; Sheldon RS, et al. 2015 Heart Rhythm Society expert consensus statement on the diagnosis and treatment of postural tachycardia syndrome, inappropriate sinus tachycardia, and vasovagal syncope. Heart Rhythm 2015;12:e41-63. Common Forms of OI • Postural tachycardia syndrome (POTS): - ≥ 40 bpm ↑ in HR in adolescents (≥ 30 bpm in adults) in first 10 min of standing or head-up tilt, with chronic OI symptoms, with no OH • Neurally mediated hypotension (NMH): - ≥ 25 mm Hg drop in BP during standing or HUT, often associated with a reduction in HR • Inappropriate sinus tachycardia (IST): - Sinus rhythm with a HR > 100 bpm at rest; similar symptoms to POTS • Low orthostatic tolerance: - Orthostatic symptoms in the absence of HR and BP changes; preliminary evidence suggests many of these individuals have reduced cerebral blood flow.
    [Show full text]
  • Evaluation of Patients with Syncope in the Emergency Department: How to Adjust Pharmacological Therapy
    medicina Review Evaluation of Patients with Syncope in the Emergency Department: How to Adjust Pharmacological Therapy Martina Rafanelli *,†, Giuseppe Dario Testa †, Giulia Rivasi and Andrea Ungar Syncope Unit, Geriatric and Intensive Care Unit, University of Florence and Azienda Ospedaliero-Universitaria Careggi, Largo Brambilla 3, 50134 Florence, Italy; [email protected] (G.D.T.); giulia.rivasi@unifi.it (G.R.); aungar@unifi.it (A.U.) * Correspondence: martina.rafanelli@unifi.it; Tel.: +39-055-7949558 or +39-333-6022642 † Contributed equally to the paper. Abstract: The rate of syncope in the Emergency Department ranges between 0.9 and 1.7%. Syncope is mostly related to a underlying reflex or orthostatic mechanism. A bradycardic or a hypotensive phenotype, may be identified. The latter is the most common and could be constitutional or drug induced. Consequently, obtaining an accurate drug history is an important step of the initial assess- ment of syncope. As anti-hypertensive medication might be responsible for orthostatic hypotension, managing hypertension in patients with syncope requires finding an ideal balance between hypoten- sive and cardiovascular risks. The choice of anti-hypertensive molecule as well as the therapeutic regimen and dosage, influences the risk of syncope. Not only could anti-hypertensive drugs have a hypotensive effect but opioids and psychoactive medications may also be involved in the mechanism of syncope. Proper drug management could reduce syncope recurrences and their consequences. Citation: Rafanelli, M.; Testa, G.D.; Keywords: syncope; orthostatic hypotension; hypotensive phenotype; hypotensive susceptibility; Rivasi, G.; Ungar, A. Evaluation of pharmacological therapy; drugs Patients with Syncope in the Emergency Department: How to Adjust Pharmacological Therapy.
    [Show full text]
  • Hemodynamic Orthostatic Dizziness/Vertigo: Diagnostic Criteria
    Kim-1 Hemodynamic Orthostatic Dizziness/Vertigo: Diagnostic Criteria Consensus document of the Committee for the Classification of Vestibular Disorders of the Bárány Society Hyun Ah Kima, Alexandre Bisdorffb, Adolfo M. Bronsteinc, Thomas Lempertd, Marcos Rossi-Izquierdoe, Jeffrey P. Staabf, Michael Struppg, Ji-Soo Kimh aDepartment of Neurology, Keimyung University Dongsan Hospital, Daegu, South Korea bDepartment of Neurology, Centre Hospitalier Emile Mayrisch, Esch-sur-Alzette, Luxembourg cDepartment of Neuro-otology, Division of Brain Sciences, Imperial College London, Charing Cross Hospital Campus, London, United Kingdom. dDepartment of Neurology, Schlosspark-Klinik, Berlin, Germany eDepartment of Otolaryngology, University Hospital Lucus Augusti, Lugo, Spain fDepartments of Psychiatry and Psychology and Otorhinolaryngology – Head and Neck Surgery, Mayo Clinic, Rochester, MN, USA gDepartment of Neurology and German Center for Vertigo and Balance Disorders, Ludwig Maximilians University, Munich, Germany hDepartment of Neurology, Seoul National University College of Medicine, Dizziness Center, Seoul National University Bundang Hospital, Seongnam, South Korea On behalf of the Committee for the Classification of Vestibular Disorders of the Bárány Society Kim-2 Corresponding author and reprint requests: Ji-Soo Kim, MD, PhD Department of Neurology, Seoul National University College of Medicine Seoul National University Bundang Hospital 173-82 Gumi-ro, Bundang-gu, Seongnam-si, Gyeonggi-do 13620, South Korea Tel:+82-31-787-7463 Email: [email protected]
    [Show full text]
  • Reductions in Cerebral Blood Flow Can Be Provoked by Sitting in Severe Myalgic Encephalomyelitis/Chronic Fatigue Syndrome Patients
    healthcare Article Reductions in Cerebral Blood Flow Can Be Provoked by Sitting in Severe Myalgic Encephalomyelitis/Chronic Fatigue Syndrome Patients C (Linda) MC van Campen 1,*, Peter C. Rowe 2 and Frans C Visser 1 1 Stichting CardioZorg, 2132 HN Hoofddorp, The Netherlands; [email protected] 2 Department of Paediatrics, John Hopkins University School of Medicine, Baltimore, MD 21205, USA; [email protected] * Correspondence: [email protected]; Tel.: +0031-208934125 Received: 7 September 2020; Accepted: 9 October 2020; Published: 11 October 2020 Abstract: Introduction: In a large study with myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) patients, we showed that 86% had symptoms of orthostatic intolerance in daily life and that 90% had an abnormal reduction in cerebral blood flow (CBF) during a standard tilt test. A standard head-up tilt test might not be tolerated by the most severely affected bed-ridden ME/CFS patients. Sitting upright is a milder orthostatic stress. The present study examined whether a sitting test, measuring cerebral blood flow by extracranial Doppler, would be sufficient to provoke abnormal reductions in cerebral blood flow in severe ME/CFS patients. Methods and results: 100 severe ME/CFS patients were studied, (88 females) and were compared with 15 healthy controls (HC) (13 females). CBF was measured first while seated for at least one hour, followed by a CBF measurement in the supine position. Fibromyalgia was present in 37 patients. Demographic data as well as supine heart rate and blood pressures were not different between ME/CFS patients and HC. Heart rate and blood pressure did not change significantly between supine and sitting both in patients and HC.
    [Show full text]